|
Cardiomyopathies
|
0.700 |
AlteredExpression
|
group |
BEFREE |
Improving skeletal muscle function without restoring dystrophin expression in cardiac tissue may exacerbate cardiomyopathy due to increased voluntary activity.
|
30972156 |
2019 |
|
Cardiomyopathies
|
0.700 |
AlteredExpression
|
group |
BEFREE |
This work demonstrates that high levels of cardiac dystrophin restored by Pip peptide-AOs prevents further deterioration of cardiomyopathy and pathology following exercise in dystrophic DMD mice.
|
25758104 |
2015 |
|
Cardiomyopathies
|
0.700 |
AlteredExpression
|
group |
BEFREE |
In conclusion, this study demonstrated that allogeneic WT-MPC-Exo transplantation transiently restored dystrophin gene expression and improved cardiac function in MDX mice, suggesting that allogenic exosomal delivery may serve as an alternative treatment for cardiomyopathy of DMD.
|
30155598 |
2018 |
|
Cardiomyopathies
|
0.700 |
AlteredExpression
|
group |
BEFREE |
The absence of dystrophin and the down-regulation of the dystrophin-associated proteins in the heart accounted for the severe cardiomyopathy in this family.
|
7635962 |
1995 |
|
Cardiomyopathies
|
0.700 |
Therapeutic
|
group |
CTD_human |
Intolerance to ß-blockade in a mouse model of δ-sarcoglycan-deficient muscular dystrophy cardiomyopathy.
|
20675662 |
2010 |
|
Cardiomyopathies
|
0.700 |
Therapeutic
|
group |
CTD_human |
Combined deficiency of dystrophin and beta1 integrin in the cardiac myocyte causes myocardial dysfunction, fibrosis and calcification.
|
18340010 |
2008 |
|
Cardiomyopathies
|
0.700 |
Therapeutic
|
group |
CTD_human |
Systemic administration of micro-dystrophin restores cardiac geometry and prevents dobutamine-induced cardiac pump failure.
|
17440445 |
2007 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Dystrophin-deficient cardiomyopathy is becoming the dominant cause of death in patients with Duchenne muscular dystrophy (DMD), but its developmental process remains elusive.
|
31611157 |
2020 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Left ventricular thrombosis and systemic emboli have been demonstrated to complicate cardiomyopathy in Duchenne and Becker muscular dystrophy (DMD, BMD).
|
10235436 |
1999 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
All dystrophin-associated proteins are decreased in abundance in the cardiomyopathic hamster heart, perhaps explaining why the cardiomyopathy is more severe than the myopathy.
|
8505286 |
1993 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Dystrophin deficiency leads to ambulation loss and cardiomyopathy.
|
30518686 |
2018 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Dystrophin-deficient Duchenne cardiomyopathy is associated with Duchenne muscular dystrophy (DMD), the most common lethal muscular dystrophy.
|
22318092 |
2012 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Although this minigene fully normalized skeletal muscle force, it only partially corrected electrocardiogram and heart hemodynamics in dystrophin-null mdx mice that had moderate cardiomyopathy.
|
29433343 |
2018 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Proteomic profiling of the dystrophin-deficient mdx phenocopy of dystrophinopathy-associated cardiomyopathy.
|
24772416 |
2014 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Thus current research efforts into the elucidation of the molecular mechanisms underlying these genetic diseases are not only directed towards studying skeletal muscle necrosis but also investigate abnormalities of heart and brain dystrophin-glycoprotein complexes in cardiomyopathy and brain deficiencies associated with muscular dystrophy.
|
9850730 |
1998 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
In conclusion, low glutathione resource hastens the onset of cardiomyopathy linked to a defect in dystrophin in mdx mice.
|
20696779 |
2010 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
GENOMICS_ENGLAND |
|
|
|
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
These findings indicate that AAV-mediated cardiac transduction with microdystrophin might be a promising therapeutic strategy for the treatment of dystrophin-deficient cardiomyopathy.
|
21451578 |
2011 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
CTD_human |
Combined deficiency of dystrophin and beta1 integrin in the cardiac myocyte causes myocardial dysfunction, fibrosis and calcification.
|
18340010 |
2008 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Dystrophin-Deficient Cardiomyopathy.
|
27230049 |
2016 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Heart failure invariably affects patients with various forms of Muscular Dystrophy (MD), but the onset and molecular sequelae of altered structure and function resulting from full-length dystrophin (Dp427) deficiency in MD heart tissue are poorly understood.To better understand the role of dystrophin in cardiomyocyte development and the earliest phase of DMD cardiomyopathy, we studied human cardiomyocytes differentiated from induced pluripotent stem cells (hiPSC-CMs) obtained from the urine of a Deuchenne Muscular Dystrophy (DMD) patient.
|
31049579 |
2020 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
These results indicate that viral infection can influence the severity and penetrance of the cardiomyopathy that occurs in the hearts of dystrophin-deficient individuals.
|
12118246 |
2002 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
The cardiomyopathy of Duchenne's muscular dystrophy and the function of dystrophin.
|
8502196 |
1993 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Although mdx mice are deficient in dystrophin, they only develop mild indicators of cardiomyopathy before 1year-of-age, making therapeutic investigations using this model lengthy.
|
22749475 |
2012 |
|
Cardiomyopathies
|
0.700 |
Biomarker
|
group |
BEFREE |
Dystrophin deficiency also gives rise to considerable complications in the heart, including cardiomyopathy development and arrhythmias.
|
30360568 |
2018 |